pulmonary regurgitation and pulmonary valve replacement...
TRANSCRIPT
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Pulmonary Regurgitation and Pulmonary Valve Replacement
in the Asymptomatic Patient Stephanie Fuller, MD, MS Assistant Professor of Surgery
Perelman School of Medicine at the University of Pennsylvania
Surgical Director, Philadelphia Adult Congenital Heart Center
A Joint Program of The Children’s Hospital of Philadelphia and Penn Medicine
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Pulmonary Regurgitation and Pulmonary Valve Replacement in the Asymptomatic Patient
Stephanie Fuller, MD, MS Assistant Professor of Surgery
Perelman School of Medicine at the University of Pennsylvania
Surgical Director, Philadelphia Adult Congenital Heart Center
A Joint Program of The Children’s Hospital of Philadelphia and Penn Medicine
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Disclosure
Co-Investigator
Medtronic Melody® Transcatheter Pulmonary Valve Post-Approval Study
CHOP IRB Number 10-007621
No financial disclosures
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STS Database Valve Procedures 2011
AVR
MVR
TVR
PVR
4,028
5,573
315
19,437 19,437
STS Adult Database 2011
160
198 546
61
STS Congenital Database 2011 – Adult only
29,353 Total Valve Replacements Pulmonary: 1.07%
965 Total Valve Replacements Pulmonary: 56.6%
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STS Database Valve Procedures 2011
AVR
MVR
TVR
PVR
4,028
5,573
315
19,437
19,437
STS Adult Database 2011
160
198 604
61
STS Congenital Database 2011 – Adult only
29,353 Total Valve Replacements Pulmonary: 1.07%
965 Total Valve Replacements Pulmonary: 56.6%
LESS THAN 1,000 PULMONARY VALVE REPLACEMENTS PERFORMED
ANNUALLY IN ADULTS
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STS Database Valve Procedures 2011
4,028
5,573
315
19,437 19,437
546
STS Congenital Database 2011 – Adult only
965 Total Valve Replacements Pulmonary: 56.6% 0
100
200
300
400
500
600
Replacement Repair Other
9.5%
Total 612
Replace 546
Repair 58
Other 8
89.2%
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1980 –
1970 –
1960 –
1940 –
0 10 20 30 40
Decade
Born with
CHD
50 60 70 80 90 100
80
50
15
5
Warnes et al. J Am Coll Cardiol 2001;37:1170-75.
Van der Velde ET et al. Eur J Epidem 2005:20:549-57.
1% D-TGA and 10% of TOF to adulthood
Epidemiology Survival to 18 yrs of Age with Complex CHD
Percent Survival to 18 Years Old
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1,300,000 –
–
–
1,000,000 –
–
–
700,000 –
–
500,000 –
–
300,000 –
–
100,000 –
0 –
1970 1980 1990 2000 2010
Adult
CHD
Patients
325,000
500,000
750,000
1,000,000
1,300,000
20,000 new patients/yr
5% increase/yr
Hoffman 1978 Kaiser
Fyler 1980 New England
Ferencz 1985 Baltimore-DC
Epidemiology
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Pulmonary Regurgitation
Residual pulmonary regurgitation is an important determinant of outcome in patients with tetralogy of Fallot as it may contribute to:
Right ventricular (RV) enlargement
Right ventricular (RV) function
Exercise intolerance
Propensity for arrhythmias
Increased risk for sudden cardiac death
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Causes of Death in Tetralogy of Fallot Survivors
Gatzoulis et al. Risk factors for arrythmia and sudden cardiac death late after repair of tetralogy of Fallot: a multicentre study. Lancet 2000;356:975-81.
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Indications for Pulmonary Valve Replacement
Gatzoulis et al. Risk factors for arrythmia and sudden cardiac death late after repair of tetralogy of Fallot: a multicentre study. Lancet 2000;356:975-81.
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Indications for Pulmonary Valve Replacement
Gatzoulis et al. Risk factors for arrythmia and sudden cardiac death late after repair of tetralogy of Fallot: a multicentre study. Lancet 2000;356:975-81.
Predictors of Sudden Cardiac Death
History
Syncope
Later age at Repair
Associated morbidity
Activity level
Hemodynamic
Enlargement of RV
Severity of PR
Severity of TR
Ventricular Function
Electrophysiologic
QRS Duration
Incidence of SVT
Sustained SVT
Khairy et al. Value of programmed ventricular stimulation after tetralogy of Fallot repair: A multicenter study. Circulation 2004:109;1994-2000.
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Indications for Pulmonary Valve Replacement
Symptoms Asymptomatic Incidental
Exercise Intolerance
Anticipation of
Symptoms
i.e. Pregnancy
Planned
Data:
Electrophysiologic
Echocardiographic
MRI
Exercise Testing
Pulmonary Stenosis
Residual VSD
Severe PA
Enlargement
Tricuspid Valve
Regurgitation
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Indications for Pulmonary Valve Replacement
Symptoms Asymptomatic Incidental
Exercise Intolerance
Anticipation of
Symptoms
Data:
Electrophysiologic
Echocardiographic
MRI
Exercise Testing
Additional Lesions:
Pulmonary Stenosis
Residual VSD
Severe PA
Enlargement
Tricuspid Valve
Regurgitation
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CMR Protocol for TOF
• Static true-FISP (Fast Imaging with Steady State Precision)
– Contiguous axial images to assess CV anatomy
• Multiplanar Reformatting – 4-chamber and short axis ventricular views for cine
– MPA and branch PA flow
• Cine Imaging
• Phase encoded velocity mapping – Aortic annulus, MPA, branch pulmonary arteries
– Stroke Volume = EDV-ESV
– Ejection Fraction = Stroke Volume/EDV
– Cardiac Output = Stroke Volume x HR
– Regurgitant Fraction =( reverse flow/forward flow) x100 *EKG gated
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Are the Indications the Same for All Patients?
• Data:
– Severe PR
– Mild – Moderate TR
– RVEF 40%
– LVEF 60%
– RVEDVi 172cc/m2
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Are the Indications the Same for All Patients?
• Data:
– Severe PR
– Mild – Moderate TR
– RVEF 40%
– LVEF 60%
– RVEDVi 172cc/m2
• What if your patient is:
– 19yo Male Sedentary
– 27yo Female planning
pregnancy
– 43yo Single mother of two
– 52yo Attorney
– 17yo NCAA athlete
– 15yo highschool athlete
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Risk of Pulmonary Valve Replacement
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Most studies suggest that restoration of pulmonary valve competency:
Decreased RV Volume and size
Improved exercise tolerance and capacity
Decreased propensity for arrhythmias
Decreased risk for sudden cardiac death
± Possible decrease in QRS duration
± Possible improvement in RV function
Indications
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Indications for Pulmonary Valve Replacement
Parameters: RV:LV Size Ratio QRS Duration Severity of TV Regurgitation Severity of PV Regurgitation RVEDV RVESV RV EF % LV EF %
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Indications for Pulmonary Valve Replacement
Parameters:
RV:LV Size Ratio QRS Duration Severity of TV Regurgitation Severity of PV Regurgitation RVEDV RVESV RV EF % LV EF %
Parameters: RV:LV Size Ratio QRS Duration Severity of TV Regurgitation Severity of PV Regurgitation RVEDV RVESV RV EF % LV EF %
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Indications for Pulmonary Valve Replacement
Parameters:
RV:LV Size Ratio QRS Duration Severity of TV Regurgitation Severity of PV Regurgitation RVEDV RVESV RV EF % LV EF %
Parameters: RV:LV Size Ratio QRS Duration Severity of TV Regurgitation Severity of PV Regurgitation RVEDV RVESV RV EF % LV EF %
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Indications for Pulmonary Valve Replacement
Kogon et al. Risk factor for early pulmonary valve replacement after valve disruption in congenital pulmonary stenosis and tetralogy of Fallot. JTCVS 2009;138:103-8.
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Indications for Pulmonary Valve Replacement
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Indications for Pulmonary Valve Replacement
RVEDV > 160mL/m2
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Thresholds for PVR
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Does PVR Improve RV Size?
Adamson et al. ICVTS 2009;9:520-7.
0
100
200
300
400
RVEDV ml
ml/m2
Pre PVR
Post PVR
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Change in NYHA Classification after Pulmonary Valve Replacement
Discigil et al. Late Pulmonary Valve Replacement after repair of Tetralogy of Fallot. JTCVS 2001;121:344-51
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Change in NYHA Classification after Pulmonary Valve Replacement
Gengsakul et al. The impact of pulmonary valve replacement after tetralogy of Fallot repair: a matched comparison. EJCTS 2007;32:462-8.
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Results after Pulmonary Valve Replacement
• Graham et al., 2008
– 93 adult patients
• Decreased RV size by 83% on echo (p<0.001)
• Systolic function increased by 36% (p=0.09)
• Ability index improved in 59% (p<0.001)
• NYHA status improved in 57%
• No improvement in arrhythmia
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Surgical Options
• Repair
– RVOT Revisions and Bicuspidization
– Valve Repair
• Resuspension
• Lengthening of Leaflets
• Replacement
– Monocusp
– Homograft Valve
– Heterograft Valve – Bovine, Porcine
– Mechanical Valve
– Percutaneous Heterograft Valve
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Valve Repair
Cowgill et al. Repair of Pulmonary Valve insudficiency Using an Autologous Monocusp. Ann Thorac Surg1986;42:587-9.ç
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Valve Repair
Cowgill et al. Repair of Pulmonary Valve insudficiency Using an Autologous Monocusp. Ann Thorac Surg1986;42:587-9.ç
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Valve Repair
Papadopoulos et al. Secondary Repair of Incompetent pulmonary Valves. Ann Thorac Surg 2009;87:1879-84
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Valve Repair
Mainwaring et al. Late Repair of the Native Pulmonary Valve in Patients with Pulmonary Insufficiency After Surgery for Tetralogy of Fallot. Ann Thorac Surg 2012;93:677-9.
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Valve Repair
Mainwaring et al. Late Repair of the Native Pulmonary Valve in Patients with Pulmonary Insufficiency After Surgery for Tetralogy of Fallot. Ann Thorac Surg 2012;93:677-9.
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Valve Repair
Pretre et al. Recycling fo the Pulmonary Valve: An Elegant Solution for Secondary Pulmonary Regurgitation in Patients with Tetralogy of Fallot. Ann Thorac Surg 2012;94:850-3.
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Valve Replacement
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Pulmonary Valve Replacement – Advanced Considerations
• Strut Position – 6/10/2 o’clock
• Valve Orientation
• Need for pulmonary arterioplasty
• Possible RV Remodeling
– Resection of Transannular Patch
– Enlargement of RVOT
• Consider future operation
– Percutaneous Valve Insertion
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Valve Replacement
Misbach et al. Pulmonary valve replacement for regurgitation after Repair of Tetralogy of Fallot. Ann Thorac Surg 1983;36:684-91
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Valve Replacement
Misbach et al. Pulmonary valve replacement for regurgitation after Repair of Tetralogy of Fallot. Ann Thorac Surg 1983;36:684-91
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Valve Replacement
Kogon et al. Adult Congenital Pulmonary Valve Replacement: A Simple, Effective and Reproducible Technique . Cong Heart Dis. 2007:2;314-8.
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Valve Replacement
Kogon et al. Adult Congenital Pulmonary Valve Replacement: A Simple, Effective and Reproducible Technique . Cong Heart Dis. 2007:2;314-8.
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Comparison of Bioprosthesis Durability
Zubari et al. Risk Factors for Prosthesis Failure in Pulmonary Valve Replacement. Ann Thorac Surg 2011;91:561-5.
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Risk Factors for Prosthesis Failure
Zubari et al. Risk Factors for Prosthesis Failure in Pulmonary Valve Replacement. Ann Thorac Surg 2011;91:561-5.
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Chen et al. Younger age and valve oversizing are predicators of structural valve deterioration after pulmonary valve replacement in tetralogy of Fallot. JTCVS 2012;143:352-60.
Risk Factors for Prosthesis Failure
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Deorsola et al.
– 4 patients @ 11 years post PVR with mechanical valve 23mm
– ¾ patients compliant with Coumadin
– F/up Echocardiogram:
• Markedly improved RV function
• Mild Dilatation
• Mild residual pulmonary gradients (10-35mm Hg) with stable RV hypertrophy
Mechanical Pulmonary Valve Replacement
Deorsola et al. Pulmonary Valve Replacement with Mechnical Prosthesis: Long -term Results in 4 Patients. Annals Thoracic Surg 2010:89;2036-8.
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Waterbolk et al.
– 27 mechanical PVRs
• Mortality – 1 early and 1 late due to complications of fatal anticoagulation related pulmonary hemorrhage
• Failure – one replaced due to fibrous tissue overgrowth
• Mean f/up is 5.5 yrs. (2mos – 18yrs)
Mechanical Pulmonary Valve Replacement
Waterbolk et al. Pulmonary valve replacement with a mechanical prothesis. Eur J Cardiothorac Surg; 2006;30(1):28-32.
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Percutaneous Pulmonary Valves
Melody™ • Bovine jugular venous valve
segment
• Platinum-Iridium Stent
Sapien™ • Bovine pericardial tissue
leaflets
• Stainless Steel Stent
23 mm 27 mm
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• Fig. 2A and 2B – Anteroposterior and lateral views of a dysfunctional right ventricle to pulmonary artery conduit demonstrating conduit stenosis and insufficiency.
• Fig. 3A and 3B – Anteroposterior and lateral views of the conduit following Melody valve insertion. A bare metal stent was introduced first to alleviate conduit stenosis. This demonstrates no residual pulmonary valve insufficiency or obstruction to forward flow.
Melody Valve Implantation
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Melody Valve Implantation
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Results of Melody Valve Implantation
Lurz et al. JACC 2011;57(6):724-31.
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Conclusions
Longitudinal assessment of pulmonary insufficiency and RV function remain critical in the medical care of the patient with pulmonary valve disease including
tetralogy of Fallot.
The number of patients requiring pulmonic valve interventions will increase.
The optimal timing of PV replacement is still unknown.
No prospective randomized trial exists in children with PR after TOF repair.
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Conclusions
Pulmonary valve repair may be a viable strategy but long term results unknown.
All choices of pulmonary valves have limited durability. With the widespread use of percutaneous pulmonary valves, earlier implantation may improve
long term results.
Considerations include:
• Durability of percutaneous valves
• Need for repeat interventions
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Conclusions
Percutaneous valves will eventually be approved for use in the native RVOT.
However, candidates will be limited by:
• Size of RVOT
• Residual hemodymanically significant lesions
– Pulmonary Artery Stenosis
– Ventricular Septal defects
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Factors influencing progression of PR after TOF Repair
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Abnormalities in Post op TOF
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Repaired TOF
• RV power output vs
RVEDV and RVESV
demonstrating
exponential decay
• Cath and CMR within
6 months of each
other
• 20% power loss
Fogel, CHOP 2010
RV Output Power Index vs. RVEDVNormalized
y = 32295x-1.20
R = 0.87
0
50
100
150
200
250
300
350
0 50 100 150 200 250 300
RVEDVNormalized
RV O
utpu
t Pow
er In
dex
RV Output Power Index vs. RVESVNormalized
y = 2168.7x-0.78
R = 0.68
0
50
100
150
200
250
300
350
0 50 100 150 200
RVESVNormalized
RV O
utpu
t Pow
er In
dex